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Lymphedema: Current Issues in Research and Management


Jeanne A. Petrek, MD; Peter I. Pressman, MD; and Robert A. Smith, PhD

Abstract
Lymphedema is a common and troublesome problem that can develop following breast cancer treatment. As with other quality-of-life and nonlethal conditions, it receives less research funding and attention than do many other areas of study. In 1998, an invited workshop sponsored by the American Cancer Society reviewed and evaluated the current state of knowledge about lymphedema. Recommendations and research initiatives proposed by the 60 international participants are presented in the conclusion section of the article, following a summary of current knowledge of the anatomy, physiology, detection, and current treatment of lymphedema. The etiology of lymphedema is multifaceted; all of the factors that contribute to the condition and the nature of their interaction have not yet been identied. To compound the problem, methods of assessing the degree of arm and hand swelling vary and are not agreed upon, and reliable methods of assessing the functional impact of lymphedema have not yet been developed.
Dr. Petrek is Director, Surgical Program, Evelyn H. Lauder Breast Center, and Attending Surgeon, Breast Service, Department of Surgery at Memorial Sloan-Kettering Cancer Center in New York City, NY. Dr. Pressman is Clinical Professor of Surgery at Albert Einstein School of Medicine and Beth Israel and Lenox Hill Hospitals, New York City, NY. Dr. Smith is Director of Cancer Screening with the American Cancer Society, Atlanta, GA. This article is also available online at www.cajournal.org.

In the absence of a cure for lymphedema, precautions and prevention are emphasized. Current treatments include elevation, elastic garments, pneumatic compression pumps, and complete decongestive therapy; surgical and medical techniques remain controversial. Elements and details of these treatments are described. (CA Cancer J Clin 2000;50:292-307.)

Introduction
Approximately 15% to 20% of breast cancer patients develop lymphedema following breast cancer treatment. This means that of perhaps two million US breast cancer survivors, after lymphadenectomy, approximately 400,000 cope on a daily basis with the disgurement, discomfort, and disability associated with arm and hand swelling. Lymphedema is among the most dreaded sequelae of breast cancer treatment. Lymphedema is a common and troublesome problem: The cosmetic deformity can not be disguised with normal clothing; physical discomfort and upperextremity disability are associated with enlargement and recurrent episodes of cellulitis, and lymphangitis may be expected in this setting. In addition to these physical symptoms, patients may experience distress caused unintentionally by clinicians whose primary focus is cancer recurrence and who may therefore trivialize lymphedema because of its nonlethal nature. For the patient, however, the appearance of arm swelling may be more distressing than living with a mastectomy, as the latter can be easily hidden while the disgCaA cancer Journal for Clinicians

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Figure

Copies of the Lymphedema monograph are available from the American Cancer Society for $9.95 by calling 1-888-227-5552.

ured arm/hand is a constant reminder of the breast cancer and a subject of curiosity to others.

ternational experts (including the authors of this article) who made recommendations for basic research, clinical practice, and public and professional education and advocacy. (See Recommendations and Research Initiatives, page 303). The workshop carefully reviewed the standard treatment of lymphedema, and faculty from each of the four main treatment schools and clinics discussed their respective techniques of manual lymphatic drainage (MLD). The entire proceedings of the meeting, including recommendations summarized in workgroup reports are available as a journal supplement (Cancer 1998;83:2775-2890) and as a separate 129-page monograph from the American Cancer Society1 (Fig.). The publication concludes with a lymphedema resource guide listing information on professional organizations addressing lymphedema, lymphedema support groups, online groups and information sources, suppliers of lymphedema pumps and garments, and schools for complex decongestive therapy, as well as a recommended reading list.

The American Cancer Society Workshop and Invited Conference on Lymphedema


The study of the incidence, etiology, and treatment of lymphedema is hampered by the decades-long course of this complication. Furthermore, lymphedema, along with other quality-of-life and nonlethal conditions, has received less research funding than have many other areas of study. To generate new attention for the problem of breast-cancer-treatment-related lymphedema, the American Cancer Society and the Longaberger Company (Newark, Ohio) sponsored an invited workshop to review and evaluate the current state of knowledge of the condition. The workshop, which took place in February 1998 in New York City, featured 60 inVol. 50 No. 5 september/october 2000

Anatomy and Physiopathology of Lymphedema


Lymph is normally cleared from tissue spaces through a network of thin-walled lymphatics, which traverse the axillary nodal basin and ultimately empty into the venous system. Lymphedema is the accumulation of protein-rich uid in soft tissues resulting from overload of these lymphatics (i.e., when lymph volume exceeds transport capabilities). Primary lymphedema refers to rare developmental abnormalities in the lymphatics and can present early or late in life. Secondary lymphedema is more common and includes among its causes surgery, irradiation, and infection (e.g., lariasis). In secondary lymphedema, lymph transport is interrupted due to physical disruption or compression of lymphatic channels.2 Patients with breast cancer de293

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velop lymphedema from surgical resection of lymphatic vessels and lymph nodes, from radiation-induced brosis around these structures, and from obstruction of lymphatics and lymph nodes by metastatic tumor. The accumulation of interstitial macromolecules raises tissue oncotic pressure, while the disruption and blockage of lymphatics raise the hydrostatic pressure within the remaining lymphatics. Both contribute to increased tissue edema. Stasis of protein-rich uid, combined with impaired immune clearance in the extremity devoid of lymph nodes, permit repeated episodes of lymphangitis and cellulitis. Such chronic inammation leads to further brosis and impairment of the affected limb. In this way, lymphedema can begin insidiously at variable periods after axillary treatment and progress from a barely noticeable condition to one involving a severely impaired limb.

rosis of the skin and subcutaneous tissue. With repeated episodes of cellulitis and lymphangitis, the skin becomes indurated, leathery, and hyperkeratotic.

Lymphedema Assessment: Physical Measures and Imaging Techniques


About 50% of patients with documented minimal enlargement (1 to 2 cm) suffer symptoms of arm heaviness.3 A mail questionnaire found that half of the patients describing lymphedema had nevertheless not reported this problem to any doctor.4 The psychosocial aspects of lymphedema, which have been unforgivably ignored in the past, were recently reviewed by Passik and McDonald.5 Women who experience pain, have lymphedema in the dominant hand, enjoy poor social support, and/or have a passive and avoidant coping style report the highest level of disability.6 Three physical measures of lymphedema7 are available, including circumferential measures at various points (with bony landmarks as references), volumetric measures using limb submersion in uid, and skin/soft-tissue tonometry in which soft-tissue compression is quantied. The traditional method for measuring lymphedema is the tape-measured arm circumference 10 cm below or 10 cm above either the olecranon or the lateral epicondyle. While seemingly straightforward, such measurements can vary according to the degree to which the tape itself constricts soft tissue. Furthermore, it is wise to measure at least one location on the lower arm and two locations on the upper arm (instead of relying on a single value), as the shape of the arm can differ before and after swelling. Measurement of the arm volume by water displacement is more accurate and results in a single value, but the technique is unwieldy and infrequently employed. Skin/soft-tissue tonometry, performed with a tension-measuring device that presses on the skin, is not a standardized
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Presentation and Progression of Lymphedema


Lymphedema is the result of a functional overload of the lymphatic system in which lymph volume exceeds transport capabilities. The build-up of interstitial macromolecules leads to an increase in oncotic pressure in the tissues, producing more edema. Persistent swelling and the build-up of stagnant protein eventually lead to brosis and provide an excellent medium for repeated bouts of cellulitis and lymphangitis. With dilatation of the lymphatics, the valves become incompetent, causing further stasis. The muscle compartments below the deep fascia, however, are spared. Lymphedema can begin insidiously at variable periods after axillary treatment. The swelling may range in severity from mild and barely noticeable in the early stages, to extreme in later stages, causing a seriously disabling enlargement of the affected limb. A brawny skin appearance develops owing to the broscle294

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procedure. Other, more sophisticated methods that are still experimental include dichromatic differential absorptiometry,8 CT scanning,9 MRI,10 and optoelectronic scanning.11 There is no standard degree of enlargement that constitutes lymphedema. Although a 2-cm difference between arms is the most common denition, such swelling could be severe in a thin arm and unnoticeable in others. In rare cases, natural variation can account for up to a 2cm greater circumference in the dominant and overused extremity.12 Thus, both arms must be measured preoperatively for an accurate lymphedema assessment. While physical measures and imaging techniques provide quantitative assessment of arm enlargement, no reliable or standard measure exists to assess the functional impact of lymphedema. Recently, some investigators have begun to explore noninvasive, and to some extent invasive, imaging techniques to diagnose and help manage lymphedema.13 Lymphoscintigraphy, for example, has been used in a preliminary way to predict which patients are at increased risk for lymphedema after axillary treatment14,15 with the hope of emphasizing prevention strategies and the value of frequent follow-up. In the future, lymphoscintigraphy, dichromatic differential absorptiometry,8 CT scanning,9 and optoelectronic scanning11 may all be used to direct management and assess results.

lated to breast cancer treatment yielded 35 reports since 1970. Data from seven of the reports with the greatest relevance for current patients (dating from 1990; ve countries) are presented in Table 1. The reports of Ferrandez et al17 and Schunemann and Willich18 have been translated from the French and German, respectively. Unfortunately, the type of breast carcinoma treatment used in the studies that are detailed in Table 1 could not be included because only a few investigators reported this important variable. (The majority of participants in the study by Lin19 and all patients in those by Werner20 and Ivens,21 underwent breast-conservation therapy.) Nevertheless, as they are all recently published reports, these seven studies should be most relevant for current patients. All reports on the incidence of lymphedema, including the seven studies highlighted in Table 1, are retrospective, and the denominator (i.e., the number of patients at risk for developing lymphedema in a particular population) is imprecise or unknown. The incidence of lymphedema in the seven selected reports varied from 6%22 to 30%.23 The report with the lowest incidence of lymphedema also had the shortest follow-up, and the same surgeon, one of its authors, operated on all of the reported patients.22

Lymphedema: Etiologic Factors


SURGERY Surgery that includes extensive removal of lymph nodes has been an integral part of breast carcinoma treatment since the end of the last century. In the standard radical mastectomy, virtually all lymphatics were interrupted and removed, along with the adjacent muscles of the chest wall, the breast, and the overlying skin, so that skin grafting was required to close the defect. Operations for breast cancer have become progressively more modest,24 and almost all studies in the past 20 years12,21,25-29 report that the inci295

Lymphedema Incidence
The incidence of lymphedema reported in different studies has varied widely as a result of variations in a number of factors, including the extent of axillary treatment, the interval between axillary treatment and measurement, methods used to dene lymphedema, and the completeness of follow-up. A comprehensive computerized search16 of the worldwide medical literature on the incidence of lymphedema reVol. 50 No. 5 september/october 2000

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Table 1 Incidence of Lymphedema Based on Reports Published Since 1990


Denition Incidence Follow up No. of Paitents Source Treatment Yrs

Year

First Author/ Journal

Country

Type of Measurement

1991

Werner 20 Radiology 19.5% 37 months* > 3 cm 6% > 12 months

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Circumference

2.5 cm

1992

England

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Ball 22 Ann R Coll Surg Engl Ivens 21 Br J Cancer N/A > 200 ml 2 yrs* 16% 10 % 2 yrs*

England

Patient Report Volumetric

1993

Lin 19 J Clin Oncol a) b) c) a) b) < 3 cm 3 cm 4 cm > 4 cm > 3 cm < 10 cm 10 cm 16% 6% 2% 14.3% 2.6%

US

Circumference

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1996

Ferrandez 17 Bull Cancer

France

Circumference

14 months*

I s s u e s

1996

Paci 23 Tumori

Italy

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5 yrs*

1997

Schunemann 18 Dtsch Med Wochenschr

Germany

Circumference

a) 4 cm b) > 4 cm < 8 cm c) 8 cm 2 cm

8.7% 13.7% 7.9% 24%

11 yrs

282 Clinic F/U 1980-1989 50 1 surgeon 1982-1990 106 Clinic F/U 1986-1990 283 4 surgeons 1988-1990 683 Clinic F/U 1994 238 Tumor Registry 1985-1986 5,868 Clinic F/U 1972-1995

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* Median time F/U Total F/U = follow-up Copyright 1998 American Cancer Society. Reprinted with permission from Cancer 1998;83:2778.

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dence rate and the degree of resultant lymphedema correlate with the extent of surgical dissection. As more nodes are excisedsuch as in sampling (retrieving three to eight lymph nodes) or in standard axillary dissection26the incidence rate of lymphedema increases in an approximately linear fashion. In two large studies,20,30 however, a relationship between extent of dissection and lymphedema could not be demonstrated, perhaps because rather small differences in extent of dissection were compared. Today, a level I-II axillary dissection is generally routine and in rare cases, if the lymph nodes are found to be positive, the dissection is extended to include level III. Attempts are now made to modify the scope of the operation to t the extent of the disease being treated. Aware of the risks of lymphedema, surgeons often carefully attempt to preserve fatty axillary tissue medial, lateral, and superior to the axillary vein, because this tissue may contain important lymphatic trunks, preferring to dissect the tissue below the vein. RADIATION THERAPY In every study that has evaluated the relationship between lymphedema and extent of surgical dissection, the addition of radiation therapy to the dissected axilla has proved to be a strong predictor of lymphedema.12,26,28,30 Therefore, if a complete axillary dissection has been carried outeven with findings of positive lymph nodes and extracapsular extensionaxillary radiation can often be avoided without high risk of axillary recurrence. Even when the intent is to irradiate only the breast (such as following lumpectomy), some radiation dosage reaches level I and even level II of the axilla, depending on the radiation-therapy technique used and on the patients anatomy. Breast radiation-therapy techniques designed specically to avoid the dissected axilla and the pathophysiology of radiation-related lymphedema were recently
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reviewed.31 For precise radiation technique, it is helpful to mark the surgical boundary with radiopaque clips, thus indicating the extent of the axillary dissection. The radiation therapist can then see the dissected area on the simulation lms and avoid it with greater accuracy. SENTINEL LYMPH NODE BIOPSY Sentinel lymph node biopsy (SLNB) (see Hsueh et al, page 279) should decrease the risk of lymphedema.32 If only one or two lymph nodes are carefully excised, it stands to reason that edema will probably not occur. The sentinel node operation is not always so limited, however. The sentinel node may, in fact, be located very high at the level of the axillary vein and the lymph trunks. Dissection at that site could theoretically result in lymphedema. The risk of lymphedema has not yet been assessed in the follow-up of patients treated with sentinel lymph node technology. Further, if axillary radiation therapy is added to SLNB, the risk of lymphedema increases. It is important to remember that in international series reporting axillary radiation therapy but no axillary surgery at all, lymphedema incidence ranged from 2% to 5%.20,21,25,30,31 Therefore, lymphedema incidence will be at least that high if SLNB and axillary radiation therapy are combined. Lymphedema after axillary radiation therapy alone was shown to develop later than that developing after combined axillary surgery and breast radiation therapy.21 Beyond these two denite factors extent of surgical dissection and radiation to the axillaexists a wide range of possible etiologic factors that have not been evaluated systematically. Some women treated decades ago with radical mastectomies and full radiation therapy, ribs thinly covered with skin grafts, did not develop lymphedema. On the other hand, there are women recently treated with modest tumorectomies, limited axillary dissections, and radiation to the breast who have developed lymphedema.
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Research to date has revealed few consistent clues to the etiology of lymphedema. One study,27 for example, reported older age at diagnosis to be a signicant factor; in another,12 this variable was unrelated to lymphedema incidence; in still others, it was curiously omitted altogether. In one study, a tendency toward lymphedema was seen when the dominant hand was on the side that had been operated on,21 but another report12 could not conrm this. Patient weight (height was not recorded) was a signicant factor in two studies,20,29 but obesity, surprisingly, was not evaluated in other studies. One study on surgical technique found a higher incidence of lymphedema with splitting the pectoralis minor muscle,25 and two studies correlated lymphedema with greater postoperative uid formation.33,34 It is surprising that the incidence of lymphedema with bilateral axillary dissection is not any higher than that after unilateral axillary dissection.16,35 In sum, etiologic factors for lymphedema have not been well studied because: 1) the course of the condition is prolonged; 2) individual patients lack long-term contact with the original surgeon and/or the radiation therapist who treated them; and, most importantly, 3) lymphedema, along with other quality-oflife issues, is perceived as less worthy of research funding.

ic factors have been studied in a limited fashion with lymphoscintigraphy.13-15 Individual patient factors, combined with axillary treatment factors, must be the main determinants of preventive strategies, notwithstanding the fact that lymphedema may occur several years after treatment. The study of events or activities in the subsequent years and decades of the patients life for the purpose of determining which are causative factors and to what degree has not been carried out. In fact, so little is known that it may be the case for some women that precautionary advice is, in fact, counterproductive, as overprotection can lead to underuse and muscle atrophy. ARM AND HAND PRECAUTIONS Arm and hand precautions are loosely based on two overarching principles: 1) The production of lymph, which is directly proportional to blood ow, should not be increased; and 2) the blockage to lymph transport should not be increased. Heat, such as that in a sauna, signicant infections, and vigorous arm exercise increase blood ow in the arm and thereby increase lymph production. Likewise, tight arm garments or infections with ensuing brosis and stenosis of lymphatic vessels may result in obstruction of lymph ow. To avoid arm swelling and/or infection, the patient should be instructed to: 1. Avoid vaccinations, injections, bloodpressure monitoring, blood drawing, and intravenous administration in the affected arm. 2. Avoid puncturing or injuring the skin in any way. Use meticulous skin and nail/cuticle care. Pay immediate attention to and use standard rst-aid care on all small or large injuries. Utilize antibiotics liberally. 3. Avoid constricting sleeves or jewelry and wear a padded bra strap to avoid constriction and pressure. 4. Avoid heat, including sunburns or tanning, hot baths, and saunas. 5. Avoid violent exercise and strenuous
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Prevention of Lymphedema
As controlling lymphedema requires daily attention, and as a cure for lymphedema has not been established, emphasis must be placed on prevention. Nevertheless, without evidence-based knowledge of etiologic factors, the list of post-treatment arm precautions is based on intuitive reasoning. It is important to remember that each woman has a congenitally different anatomy, which, like the rest of the vascular system, is probably uniquely prone to degenerative conditions. Such anatom298

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exertion. Consider vigorous aerobic arm exercise only when the arm is supported by compression garments. There are no data to govern any of these recommendations. The nding that no increase in risk of lymphedema was noted in women who had had bilateral axillary dissection compared with those who had had unilateral axillary dissection16,35 calls into question the implication that blood drawing, intravenous administration, blood-pressure monitoring, and injections hasten the development of lymphedema. Data for any of the other arm and hand precautions are even more speculative. On the other hand, breaking the skin barrier, even during medical procedures, could predispose to infection, and blood-pressure monitoring could cause injury. Lymphoscintigraphic techniques are now being used to study the lymphatics of the upper limb after axillary treatment and may help to provide answers regarding arm and hand precautions.13 Dynamic as well as static images may be obtained at various levels in the affected arm and under various standardized conditions (during and/or after various periods of rest, during and/or after exercise, etc.). Such research is desperately needed. All patients are currently instructed in the same arm and hand care precautions; however, these precautions may be too severe for those at low risk, while not aggressive enough for those at the greatest risk. Furthermore, as lymphedema may occur even several decades36 after axillary treatment, patients are admonished to follow these demanding precautions for the remainder of their lives. LYMPHEDEMA TREATMENTS Therapeutic nihilism (i.e., no treatment at all) for lymphedema is deplorable, although quite common. All too often, a woman is told that she should be thankful to be alive and that she must learn to live with it. The fact that the average clinician is ill prepared to both detect and
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recognize early signs of lymphedema must be remedied, as data suggest that the sooner the treatment is started, the smaller the amount of treatment required to prevent further progression, and the better the ultimate result. The treatment of established lymphedema varies from doing nothing at all to pursuing a host of aggressive surgical procedures, as was particularly the case in the past. Between these two extremes lie various combinations of conservative treatments, the most important of which are elevation, the use of compression garments, centripetal massage and exercises, the use of pneumatic compression devices, and a program of complete (or complex) decongestive physiotherapy, known as CDP. 1. CDP CDP has been widely available in Europe for many years. This therapeutic approach takes into account the fact that lymphedema exists in an entire body quadrant, although its effects are most distressing in the arm or hand, and includes skin care, gentle specic massage, known as manual lymphatic drainage or MLD, low-stretch multilayer compression bandaging (followed by a tted compression garment when edema is reduced), and therapeutic exercises with the garment or bandages in place. The 1998 American Cancer Society Workshop on Breast Cancer TreatmentRelated Lymphedema included a review of the modications and features of the various CDP programs described by Vodder,37 Leduc,38 Foldi,39 and CasleySmith.40 Although the principles followed are the same for each school, the massage techniques vary somewhat in terms of the degree of pressure and motion applied and the timing of strokes. Additionally, the Leduc technique uses low, intermittent pneumatic pressure (< 40 mm Hg) pumps, and the Casley-Smith group uses benzopyrone medication. CDP must be performed by skilled, specially trained therapists. A typical
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Table 2 Complex Decongestive Therapy (CDT) Phase I: Treatment1 to 4 weeks


Meticulous skin and nail care Manual lymphatic drainage Low-stretch multilayer bandaging Physical therapy in bandages

Phase II: Maintenance


Meticulous skin and nail care Low-stretch multilayer bandages worn overnight Prescribed exercises in bandages [Surgical support garments (30-50mm Hg) for ongoing control]

American program includes four phases. During Phase I (the treatment phase), the patient is given one or two 75- to 90minute treatments daily over a period of one to four weeks. In Phase II (the maintenance phase), the patient maintains and optimizes the results by applying some of the techniques learned in the treatment phase, as well as by wearing an elastic sleeve during the day, bandaging the affected limb overnight (as described below), and exercising for 15 minutes a day while wearing the bandages. Phase II is continued indenitely or until the limb no longer swells. Phase I of CDP treatment consists of four steps: 1. Meticulous skin and nail care, which can optimize the supple normal texture. 2. MLD or manual lymph therapy (MLT), a delicate massage technique that stimulates lymph vessels to contract more frequently, directing and channeling uid toward adjacent, functioning lymph basins. Manual lymph drainage begins with stimulation of the
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lymph vessels and nodes in unaffected and opposite basins (neck, contralateral axilla, ipsilateral groin). Edema uid and obstructed lymphatics are made to drain toward functioning lymph basins across the midline of the body, down toward the groin, over the top of the shoulder, around the back, etc. Finally, in segmental order, massage of the involved trunk, the shoulder, upper arm, forearm, wrist, and hand. 3. Multilayer low-stretch bandaging is done immediately following manual lymph drainage. Bandages are wrapped from the ngertips to the axilla with maximal pressure distally and less pressure proximally. Many layers of minimally elastic cotton bandages are used, beneath which layers of foam rubber padding are inserted to ensure uniform pressure distribution or to increase pressure in areas that are particularly brotic. 4. The bandaged patient is next guided through exercises involving active range of motion with the muscles and joints functioning within the closed space of the bandaging. Isometric exercise is generally avoided. The steps involved in the maintenance phase of CDP are shown in Table 2. After volume reduction has been accomplished, well-tted compressive garments continue ongoing control of edema. It is generally not helpful to t the garment prior to volume reduction. The patient should be re-measured and the garment replaced every three months. The patient and the patients family will have been trained to continue the maintenance program at home. Follow-up visits to the center usually take place at sixmonth intervals for measurements and continued instruction about different components of the program. A recent short-term study of 28 women randomized to either the Vodder technique or to sequential pneumatic compression (pumping) favored the CDP program.41 Low-stretch compresCaA cancer Journal for Clinicians

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sion bandaging, as in Phase I, has also been used alone (without MLD) with some good effects.42 Although this burgeoning technique appears more successful than other modalities in reversing lymphedema, the availability of patient services and treatment centers that can utilize CDP is limited. Representatives of the four schools have recently reviewed and discussed issues of patient accessibility43 and professional education for physical therapists.44 Ongoing training of therapists and physicians is offered at several centers to teach CDP techniques. (The theories and clinical applications of the four schools of manual lymphatic technique are reviewed in the American Cancer Society monograph by their respective faculties.37-40) 2. Elevation and Elastic Garments The elements of elevation and some details of the use of elastic garments are presented here.45 Although elevation may be helpful in reducing swelling from lymphedema through the use of gravity, it is impractical. A patient with lymphedema should be tted with an elastic sleeve from wrist to axilla if the edema is mild; if the edema is moderate, the tting should take place after the reduction of swelling. A separate, removable gauntlet or handpiece allows the patient to wash her hands without removing the entire sleeve. The physician who prescribes the support garment should be aware of the different products that are available and should order a garment in the proper compression class. These classes are: I. 20 to 30 mm Hg II. 30 to 40 mm Hg III. 40 to 50 mm Hg IV. 50 to 60 mm Hg For upper extremity lymphedema, a Class II or III support is generally required. The person measuring the lymphedematous arm and hand should be trained in tting such garments and in instructing patients in their proper application. Too often, however, this task is left
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to a clerk in a surgical supply store who lacks specic training. A statistically signicant reduction in edema has been reported in women who wore compression garments for six consecutive hours per day.46 Using these garments during exercise, physical activity, and air travel is recommended. 3. Pneumatic Pumps The older intermittent, single-chamber, nonsegmented compression pumps provide even pressure throughout the treated arm; however, they also allow backow of the lymphatic uid, which may cause an increase of uid in the distal arm. Newer devices have multiple chambers and can provide sequential compression. The standard sequential system is a multichamber pump that delivers the compression at the same pressure in each garment section from distal to proximal tissues. The gradient sequential system delivers pressures that differ by approximately 10 mm Hg between each chamber, with the higher pressures delivered to the distal chamber. A minimum of one hour per pumping session is required, and lower pressures for longer periods are more effective than higher pressures for shorter periods. The arm should be elevated during pumping. Women with lymphedema should not simply be dispatched to the medical supply house with a prescription and instructions to buy a pump and to begin using it after reading the accompanying instructions. Individualized, tailored pumping programs are based on empirical knowledge of what will work for each individual patient. Recommendations for the use of a particular pump and program should be based on measurable efficacy and tolerability, as evidenced by serial assessment in that patient. The patient is then instructed about the limitations and use of her pump before she is placed on a home program. Several controlled studies47-51 have reported reductions in lymphedema with the use of various devices. Although the
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use of machinery for the pumping action on the arm lymphatics is theoretically attractive, pumping has not been as effective clinically as had been hoped. While CDP is quite successful for lymphedema, constraints created by the need for experienced personnel and by time requirements for treatment limit its availability. It has been hoped that pneumatic compression devices or pumps could duplicate the benecial effects of massage.45 Such pumps can force protein-rich edema uid toward the shoulder, an area already congested, but not, however, through the axillary blockage; lymphedema involves the whole quadrant of the ipsilateral trunk, the area that the obstructed axillary channels would normally drain. In particular, pneumatic compression therapy appears less useful in advanced lymphedema because of skin thickening and brosis. For recent reviews of the rationale for and controversies about pumps, see Brennan and Miller45 and Rinehart-Ayres.52 4. Surgical Treatment Operations with intent to cure lymphedema are cited here mainly for historical interest. Numerous surgical procedures have been proposed and attempted in the treatment of chronic lymphedema yet none has been clinically successful. Surgical approaches can be divided into two categories, physiologic and reductive. Physiologic approaches aim to restore lymphatic ow to the limb either by reconstruction of lymphatic channels or by bridging lymphedematous tissue to areas with normal lymphatics, usually by direct microsurgical anastomosis of several lymphatics to veins. Reductive approaches simply remove excess tissue and edema to reduce the limb to a more functional size. These operations include removal of skin and subcutaneous tissue followed by skin grafting (Charles procedure), or staged subcutaneous excision beneath skin aps (Sistrunk procedure).

Very recently, short-term success with liposuction has been noted in Sweden by one group,53-55 although the long-term efcacy of the procedure is not known. The state of knowledge about surgical procedures for lymphedema was recently reviewed by Brennan and Miller.45 5. Medications Diuretics are not effective in high-protein edemas such as lymphedema. Although the diuretics can temporarily mobilize water, the osmotic pressure from the increased protein in the interstitial space causes rapid reaccumulation of edema. Benzopyrones belong to a group of drugs that include the bioavonoids and the coumarins. The former occur widely in nature, especially in fruits and vegetables. Benzopyrones improve chronic lymphedema56-57 by stimulating macrophage activity for increased proteolysis and, thereby, for the removal of stagnant, excess protein in the tissue spaces; this results in decreased oncotic pressure and edema uid. Several European and Australian researchers58-62 have experimented with 5,6-benzo--pyrone and reported that it produces a slow reduction of lymphedema. Their centers include these drugs in physical rehabilitation programs. The benzopyrones may cause liver toxicity, and deaths have been reported. In 1993, in an Australian study, a randomized, double-blind, placebo-controlled, crossover trial of 5,6-benzo-pyrone demonstrated its efficacy.63 Although the effect was mild, it was statistically signicant. More recently, however, a larger number of lymphedematous breast cancer patients participated in an American multicenter study of similar study design led by the Mayo Clinic. In this study, the benzopyrone showed no value beyond the placebo effect.64 Moreover, 6% of the study subjects had worrisome elevation of liverfunction tests.

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American Cancer Society Workshop on Breast Cancer Treatment-Related Lymphedema: Recommendations and Research Initiatives
Approximately 60 invited participants were organized into ve concurrent workgroups, each of which focused on a dimension of the current challenge of lymphedema, and each of which was charged with issuing recommendations for research, clinical practice, public and professional education, and advocacy. Workgroup reports and recommendations were published as part of the proceedings of the conference and are summarized here. Workgroup I: Treatment of the Axilla with Surgery and RadiationPreoperative and Postoperative Risk Assessment Lymphedema of the arm is caused by treatment of the axilla with surgery or radiation, and risk of lymphedema is increased when the axilla is treated with both modalities. Recommendations to reduce risk of lymphedema without compromising the fundamental intent of breast cancer treatment included: 1. Avoidance of axillary lymph node dissection for patients with low-risk lesions (i.e., ductal carcinoma in situ or certain T1 lesions) 2. Support for continued research on lymphatic mapping and SLNB, and for training and credentialing in these new techniques. Although these new techniques appear to offer great potential to reduce the risk of lymphedema in patients with lower-risk lesions, patients undergoing treatment with SLNB should be informed of the lack of long-term follow-up with these techniques and, ideally, should be enrolled in a prospective study. 3. For patients who must be managed with standard treatment, painstaking care in the technical aspects of surgery itself and radiation planning can reduce the risk of lymphedema. 4. Research is needed on pre- and post-imaging studies (e.g., lymphoscintigraphy) to evaluate the status of the axillary lymph nodes and lymphatic drainage of the arm and breast. 5. Include assessment of symptoms associated with early signs of lymphedema in routine follow-up after breast cancer treatment. All clinical trials of breast cancer treatment, such as chemotherapy assessment, should include lymphedema assessment as an additional endpoint.65 Workgroup II: Patient EducationPre- and Post-treatment Most breast cancer patients do not appear to be informed about the potential for lymphedema as a consequence of treatment for breast cancer. Pre- and post-treatment education is needed, as early signs of lymphedema may not be recognized, leading to delays in treatment and potentially to irreversible progression. Furthermore, although little is denitely known about activities or trigger events associated with lymphedema onset, patients should be fully informed so that risk-reducing behavior can be adopted. Treatment advances may offer women opportunities for informed decisions about breast cancer treatment based in part on risk of subsequent lymphedema. Informed decision-making must take place when these treatment options exist. Specic recommendations included the following: 1. Verbal and written pretreatment education on the risk of lymphedema should be introduced into discussion of breast cancer treatment options; written material should be culturally sensitive and evidence-based. 2. Post-treatment education should stress the importance of recognizing early signs of lymphedema, include information on hand and arm precautions, and offer practical advice for avoiding situations associated with lymphedema risk. Advice should be offered about what to do if these situations are encountered (i.e., a wound on the affected arm). 3. Patients who develop lymphedema should be evaluated comprehensively by experienced professionals and be fully informed about treatment options, management of acute lymphedema, and selfmaintenance of stable but chronic forms of lymphedema.66 Workgroup III: Diagnosis and Management Although lymphedema is a prevalent disorder, there is a tendency toward therapeutic neglect of lymphedema following treatment for breast cancer. Second, while there are different therapeutic approaches to lymphedema, consensus has not been reached on whether one approach is preferable to another, or under what circumstances one approach may be more appropriate than another.

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Chronic lymphedema is best treated by specialists using a multimodal approach of decongestive lymphatic therapy, which includes proper skin care, manual lymphatic therapy, multilayered low-stretch bandages, and exercise. After arm-volume reduction, ongoing control of edema must be maintained through properly tted compression garments. Another modality that may be used in addition to decongestive lymphatic therapy is intermittent compression pumps. The workgroup recommended : 1. All patients treated for breast cancer should be assessed for signs and symptoms of lymphedema at an early interval following completion of healing from breast cancer therapy (within the rst 12 weeks). Clinicians should pay attention to physical signs of lymphedema as well as to the patients subjective awareness of symptoms, as the latter may reveal early signs of an underlying pathology. 2. Research should focus on the development of screening modalities predictive of lymphedema, such as enhanced lymphoscintigraphic techniques used to measure degrees of lymphatic dysfunction. 3. Investigation of the importance of early detection and aggressive intervention for reducing severity and progression of lymphedema should be undertaken. 4. Research to determine the relative efficacy of each component of a comprehensive treatment program, including optimal timing of application.67 Workgroup IV: Lymphedema Treatment ResourceProfessional Education and Availability of Patient Services Health care providers are not sufficiently informed about lymphedema, may not recognize early symptoms of the condition, and generally are uninformed about treatment options and the availability of treatment in their communities. Because of the pivotal role played by health care professionals in terms of informing and meeting the needs of patients, recommendations relate to the development and costeffectiveness of both professional education and patient interventions. These included: 1. Development of clinical-practice guidelines focusing on professional education, lymphedema recognition, and intervention strategies in patients treated for breast cancer;
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2. Promotion of professional education focused on lymphedema, including grand rounds, continuing medical education, and greater attention to lymphedema in medical textbooks and graduate medical education; 3. Establishment of a multidisciplinary task force to establish certication guidelines for specic treatments and treatment facilities; 4. Expansion of the number of facilities available to treat lymphedema, and development of a resource guide for lymphedema clinical services; 5. Development of cost/economic analysis of the burden and treatment of lymphedema.68 Workgroup V: Collaboration and Advocacy Meeting the current and future challenge of lymphedema will be accomplished faster and more efficiently if organizations work together and address a common mission. Collaboration has the potential to reduce duplication, maximize resources, encourage greater innovation, and hasten progress. A common advocacy position among leading and inuential organizations can also be more persuasive and more effective in changing policy than uneven and uncoordinated efforts. The workgroup focused on the need for collaboration and called for the inclusion of representatives from all advocacy positions (including the American Cancer Society, the National Lymphedema Network, the National Alliance of Breast Cancer Organizations, the Susan G. Komen Foundation, Y-ME National Breast Cancer Organization, Arm-in-Arm, and Bosom Buddies) in future initiatives. Collaborative efforts should focus on patient and provider education, standards for informed consent and information, and legislative and patient advocacy. A workgroup should be established to develop a model for insurance coverage of lymphedema treatment services; the provision of coverage was advocated for state-of-the-art treatment by third-party payers. The workgroup stressed the need for effective advocacy for standards of treatment and management of lymphedema, calling for collaboration within the medical and scientic community to establish the evidence upon which future advocacy and collaborative efforts could be built.69

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Conclusion
Lymphedema is an important health problem for many women who have been treated for breast cancer. It is especially dispiriting to women who have been assured that their prognosis is excellent and that reconstruction will allow them to continue their lives with few visible reminders of their disease. For many women, lymphedema is not only a debilitating condition, but also a daily reminder of the health care systems failure to educate them appropriately and to respond effectively to their condition. Many of the recommendations for addressing the challenge of lymphedema are interrelated and are thus dependent on concomitant and synchronous progress in the development of a solid research base, treatment modalities for the reduction of risk of lymphedema, improvements in the clinical response to symptoms of lymphedema, improvements in provider and patient education, and changes in health care policy. In the interim, patients and health care providers must be fully appraised of what we know and what we do not know about breast-cancer-treatment CA related lymphedema.
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CERTIFIED THERAPISTS, such as physical and occupational therapists, typically plan rehabilitation interventions, generally in concert with a prescribing physician. Other individuals, including nurses and licensed massage therapists, may employ these techniques as well. Therapists should also identify and address pain, limitations in range of motion, and impaired activities of daily living. For mild lymphedema (resolves completely overnight): 1. Counsel: Use arm/hand precautions; normalize body weight; employ meticulous arm/hand care. 2. Elevate; employ centripetal selfadministered massage. 3. Use compression garment, particularly during work, exercise, and air travel. For moderate and severe lymphedema: (referral to a rehabilitation center recommended) 1. Counsel: Use arm/hand precautions; normalize body weight; employ meticulous arm/hand care. 2. Elevate; employ centripetal selfadministered massage. 3. Use compression garment. 4. Employ complete decongestive physiotherapy (CDP) program AND/OR intermittent pneumatic compression devices under guidance of a therapist.

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